POS0216 CARDIAC SARCOIDOSIS: AN UNDERDIAGNOSED, LIFE-THREATENING YET TREATABLE DISEASE
Authors: P. Webster, Y. Rochlani, K. Loupasakis
Journal: Annals of the Rheumatic Diseases (2022)

Background Cardiac sarcoidosis (CS) is potentially life-threatening and it typically causes heart failure, ventricular arrhythmia, AV block or sudden death. CS has been reported to account for 25% of sarcoidosis patient deaths, thus highlighting the importance of early diagnosis and treatment. Diagnostic challenges and lack of a unified screening approach make it difficult to estimate its prevalence. Immunosuppression is the mainstay of treatment, but there is paucity of data on optimal dose, duration and efficacy of steroids and synthetic (s) or biologic (b) disease-modifying anti-rheumatic drugs (DMARDs). Objectives To describe the demographic and clinical characteristics of our CS patients, and to study their clinical response to steroids, synthetic and biologic DMARDs. Methods A retrospective cohort study with a prospective ongoing phase was conducted. ICD-10 codes were used to identify patients over 18 years old with sarcoidosis and CS followed at Montefiore Medical Center from 2017 to 2021. The following variables were recorded: demographics (age, sex, ethnicity), BMI, smoking status, presenting manifestations, histopathology (cardiac and extracardiac), imaging, treatment (corticosteroids and s/bDMARDs), dose and duration of therapy, steroid-sparing effect, response to treatment based on clinical and imaging improvement (echocardiogram, cardiac MRI, PET). Results 220 patients with sarcoidosis were identified. 154 (70%) female and 124 (56.4%) Black. 42 out of 220 (19.1%) met diagnostic criteria for CS 1,2 . Mean age of CS patients was 61.6 years. 23 (54.8%) were male and 24 (57.1%) Black. Heart failure (54.8%), arrhythmias (47.6%) and heart block (28.6%) were the most common presenting manifestations. 28.6% had isolated CS. 5 (11.9%) underwent endomyocardial biopsy and 2 of them (40%) had histopathologic evidence of CS. Mean left ventricular ejection fraction (LVEF) was 40.6 % on echocardiogram, and 29 patients (69%) had PPM/ICD placed for arrhythmia prevention. 35 (83.3%) underwent cardiac MRI, 37 (88.1%) cardiac PET scan and 23 (62.3%) had a follow up PET. 33 (78.6%) CS patients were treated with corticosteroids for a mean duration of 17.3 months. 21 of them were also treated with sDMARDs and 3 were subsequently switched to bDMARDs (Table 1). Mean prednisone dose at 12 months was 4.8mg, and 11 patients (33%) were tapered off corticosteroids. Table 1. Treatment of cardiac sarcoidosis Agent Treated with DMARDs(N=21)* Duration of treatment(months, x̄) Max dose(mg, x̄) Prednisone dose at 12 months(mg, x̄) Response to treatment Methotrexate (Weekly ) 16/21(76.1%) 16.0 16.9 3.67 14/16(87.5%) Mycophenolate mofetil (Daily ) 6/21(28.6%) 16.9 2000 5.1 3/6(50%) Hydroxychloroquine (Daily ) 1/21(4.8%) 17.5 400 5 1/1(100%) Infliximab (q 8 weeks ) 1/21(4.8%) 6.0 5 (mg/kg) 5 1/1(100%) Adalimumab (q 2 weeks ) 2/21(9.5%) 10.0 40 7.5 2/2(100%) *2 patients switched from MTX to MMF and 3 from sDMARDs to bDMARDs Conclusion In our sarcoid patient population, the prevalence of CS is 19.1%, which is higher than previously reported in the literature, suggesting CS may be underdiagnosed. There was a greater proportion of male patients in the CS population (54.8%) than the generalized sarcoidosis population (30%). This indicates that males with sarcoidosis may be more likely to have cardiac involvement. Patients treated with s/bDMARDs had a high response rate as reflected by clinical and imaging improvement. Steroid-sparing effect is suggested by the low prednisone dose at 12 months (Table 1). Methotrexate was the most commonly used DMARD (76.2%). Randomized clinical trials are needed to establish robust guidelines for the treatment of CS. References [1]Birnie, David H., et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart rhythm 11.7 (2014): 1304-1323. [2]Terasaki F, Yoshinaga K. New guidelines for diagnosis of cardiac sarcoidosis in Japan. Ann Nuclear Cardiol 2017;3:42–5. Disclosure of Interests None declared